adrenal glandCorticalorganization structureIt can be divided into three layers, spherical zoneFascicular bandAnd reticular tape.Globular zoneGlandular cellArranged in a short ring or ball.This layer is thin and mainly secretesMineralocorticoidAldosterone is mainly used in human.Fascicular bandLocated in the middle of the cortex, the gland cells are arranged in bundles perpendicular to the surface of the gland.This layer is thick and forms most of the cortex.The reticular zone is located in the innermost layer of cortex, and the glandular cells are arranged irregularly.Fascicular bandIt secretes glucocorticoid with the reticular zonecortisolMainly, reticular zone also secretes a small amountsex hormone。Each layer secretes differenthormoneIt is mainly because the enzymes that promote hormone synthesis contained in each layer of cells are different, thus producing different stimulationsEnzyme reactionAlthough the substrates are the same, the hormones synthesized are different.Such as congenital or acquired causesEnzyme deficiency, can cause someCorticosteroidsInsufficient synthesis and secretion.
adrenal glandThe cortex is thick, located in the surface layer, accounting for about 80% of the adrenal gland. From outside to inside, it can be divided into three parts: the zona glomerulosa, zona fasciculata and zona reticularis.
2、Fascicular band：It accounts for about 78% of the cortical thicknesspolygonThe cells are arranged in bundles.The cells are large in size, with light staining of nuclei, and located in the center.The cytoplasm is full of fat drops. In the ordinary staining specimen, the fat drops are dissolved, leaving many small vacuolesFascicular bandThe cells are foamy.Under the electron microscope, the smooth endoplasmic reticulum is much more than the spherical zone, and it often surrounds fat droplets andmitochondrionThe arrangement and rough endoplasmic reticulum are also relatively developed.The cells in this zone secrete glucocorticoids, mainly represented by cortisone and hydrocortisone, which regulate sugar, fatproteinMetabolism of.
3. Mesh belt:It accounts for about 7% of the thickness of cortex. It is close to medulla, and the cells are arranged in irregular strips and interlacedNetworking。Cellular comparisonFascicular bandIs small, the nucleus is also small, the staining is deep, and the cytoplasm is weakAcidophilic。It contains a small amount of fat drops and more lipofuscin.Under electron microscope, there are a lot of smooth endoplasmic reticulum in the cells of this zone.Cell secretion of this zoneandrogen, but the secretion is less, which is of little significance in physiological conditions.
adrenal glandCorticosteroids are the general name of various hormones secreted by the adrenal cortex.according toAnimal experimentAnd pairadrenal glandClinical observation of patients with functional disorder, knowing that the adrenal cortex secretesLife activitiesThere are two categories of important relationshipshormone, i.eMineralocorticoidandGlucocorticoidAt the same time, it also secretes a small amount ofsex hormone。MineralocorticoidIt plays the role of sodium retention, water retention and potassium excretion for human body, and maintains human normalWater salt metabolism, body fluid volume andOsmotic equilibriumThey play an important role.GlucocorticoidClass includes cortisone（Cortisol）And hydrocortisone（cortisol）Etc.This kind of hormone affects sugarproteinandFat metabolismBoth have influence, and the main role is to promoteProteolysisharmonize the liverGluconeogenesis。When the supply of sugar in food is insufficient (such as hunger),GlucocorticoidIncreased secretion will promote muscle andconnective tissueEtcproteinAnd inhibit muscle pairsamino acidTo enhance liver gluconeogenesis and promoteliver glycogenIt is decomposed into glucose and released into the blood to increase the source of blood sugar. The blood sugar level can be maintained, so that the energy required for brain and heart tissue activities will not be lacking.Used as a drug, in large dosesGlucocorticoidAnti inflammatory, anti allergicAntitoxinFunction, anti shock and inhibitionimmune reactionIt is widely used in medicine, but there are also side effects that cannot be ignored.Normal adults,adrenal glandThe cortex also secretes a small amountsex hormone, but the effect is not obvious.Whenadrenal glandCortical certainCell proliferationOr tumor formationsex hormone(MainlyAndrogen）Secretion increases a lot. Male patients will have hair clumps, while female patients will show masculinity.
ICorticosteroidscombinationAdjuvant therapy: (1) Outbreakhepatitis B: Prednisone oral or dexamethasoneIntravenous dripMay alleviate symptoms and jaundice, or evenAcute liver failureMitigation.（2）chronic hepatitisPrednisone alone or in combination with other immunosuppressants is still a routine therapy for chronic hepatitis in foreign countries until the 1970s. Some randomized controlled clinical trials have proved that prednisone can prolong the survival period and reduceCase fatality rate。But inHBsAgAfter the establishment of the detection method, it is found that the previously reported effective treatment group contains mostly HBsA8 (-) cases, most likelyAutoimmune hepatitis。
2、 AndinterferonOfCombined treatment: During prednisone treatmentImmunosuppression，serumtransaminaseIt is often reduced, and it is usually within 1-2 months if the drug is stopped suddenlyhepatitisAggravate, and stimulateVirusesImmune clearance.HBV during this periodDNAAnd DNAp serum levels decreased, even turned negative;Then there was serum anti HBe conversion and clinical remission.Therefore, it is believed that high-dose short course prednisone treatment mayChronic hepatitis BEffective;However, it may cause serious deterioration of the disease, or evenFulminant hepatic failureThe.short-rangeCorticosteroidsIt is also not suitable for general chronic hepatitis B treatment as long term therapy.short-rangeReboundThe treatment is only suitable forAntiviral drugsJoint application of.
(1) Treatment scheme: prednisone 40mg, 30mg and 20mg/d for 2 weeks at an interval of 2Weeks laterIFN α treatment.Children received lmg/(kg · d) for one month, and also stopped for two weeks, waiting for immune rebound to start IFN α treatment.
(2) Treatment effect: clinical rebound（serumElevated transaminase）The rate is 40% - 70%.Chronic HBV infection in China often has a certain degreeImmunotoleranceTherefore, the effect rate of combined treatment is higher than that of single treatmentInterferon alphaIs significantly higher.A considerable number of patients who have been treated with single IFN α for low effect and no effect can obtain therapeutic effect by changing to combined treatment.
(3) Therapeutic mechanism: prednisone/IFN α combination therapy deeply affects cellsSubgroupDistribution and virus replication.In healthy people'sPeripheral bloodOf T cells,CD4+About 40% of cells, slightly more than 20% of CD8+cells, and nearly 2% of CD4/CD8 cells;Patients with chronic hepatitis BCD8+T cellsThe ratio of CD4/CD8 was significantly decreased.During the treatment with prednisone, the CD4/CD8 ratio increased significantly due to the significant decrease of CD8+cells, accompanied by the increase of serum HBV DNA level;During the treatment with IFN α, the CD4/CD8 ratio increased again due to the increase of CD4 'cells, accompanied by the decrease of serum HBV DNA level.Therefore,hormoneAfter drug withdrawal, CD8+cells rebound after inhibition, and IFN α promotes CD4+cell proliferation, combination therapy may be performed by enhancingcellular immunityAnd obtain curative effect.
（4）Adverse reactions: Yellow gangrene and worsening symptoms may occur.The incidence of liver decompensation in rebound patients is partly noncirrhosisClinical rebound occurs more frequently in patients, but once clinical rebound occurs in patients with cirrhosis, the relative risk of decompensation is 16 times higher than that in patients without cirrhosis.
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1. (1) RightThree nutrientsIntermediate metabolismFunction ofCarbohydrate metabolismBoth "open source" and "cost saving": on the one hand, promoteProteolysis, makeamino acidTransformed intoGlycogen；On the other hand, there is confrontationinsulinAnd inhibit the effect of peripheral tissue onglucoseMake use of, make blood sugar rise.Excessive glucocorticoid can cause a significant increase in blood sugar, which may causesteroidnaturediabetes。Patients with recessive diabetes should be cautious when using glucocorticoid clinically.The effect of glucocorticoid on fat in different parts is different.limbadipose tissueThe increased decomposition will increase the fat synthesis in abdomen, face, shoulders and back.Adrenocortical hyperfunctionSometimes or after taking excessive glucocorticoidFull moon face, buffalo back and“Centripetal obesity”Isomorphic features.
(2) YesWater salt metabolismEffects of glucocorticoid: glucocorticoid also has effects on water and salt metabolism, which mainly affects the drainage of water. In case of lack, drainage will be difficult.It may beglomerulusOfFiltrationyesPermissive effect。
(4) Effect on muscles: nail removaladrenal glandAnimalisticskeletal muscleRelaxation and weakness, supplement of glucocorticoid can makeMuscle strengthRecovery;However, too much glucocorticoid promotesProteolysis, makeproteinThere is no balance between the decomposition and renewal of. The decomposition is more than the synthesis, and the muscles are weak.
(6) Role in stress response: all harmful stimuli in the environment, such asanaesthesia, infection, poisoning, trauma, cold, fear and other factors affect the body, causing a series ofphysiological functionChanges to adapt to the above harmful stimuli are called stress reactions.In this reaction, ACTH secretion increased immediately,GlucocorticoidSecretion also increased accordingly.Glucocorticoids can enhance the stress ability of the body, but its mechanism is still unclear.In stress response, sympathetic-Adrenal medullaThe system also participates in activities.Adrenal cortical insufficiencyWhen encountering harmful stimulus, the body's stress response will be weakened, and it is easy to die.
（7）Pharmacological effect: High doseGlucocorticoidIt will cause pharmacological effects, mainly including anti-inflammatory, anti toxic, anti shock and anti allergic effects, which will be detailed inpharmacologyAs described in.
2. Secretion regulation glucocorticoid
Secretory regulationGlucocorticoidThe secretion ofStress stateThe secretion ofAdenohypophysisACTH control.After removing the adenohypophysis,adrenal glandThe fascicular zone and reticular zone of cortex atrophy,GlucocorticoidIf ACTH is supplemented in time, the atrophic tissue and secretory function can be recovered.It shows that ACTH promotesFascicular bandAnd the development and growth of reticular zone, and stimulate them to secrete glucocorticoid.ACTH secretion is also affected byhypothalamusCRH control of.ACTH is one, including 39amino acidPeptides of.Its natural secretion shows a circadian rhythm.Before waking up in the morning every day, the secretion reached its peak, and then gradually decreased, and then significantly decreased after falling asleep at night. At midnight, the secretion reached its lowest point, and then gradually increased.It is believed that this rhythm may be affected by the hypothalamusbiological clockControl of.Some people think that CRH secretion also has this rhythm, forming hypothalamus glandthe pituitary-adrenal glandCortical axis.Glucocorticoids in blood have effects on CRH and ACTHnegative feedback regulation, is a closed loop.In bloodGlucocorticoidIn case of excessive secretion, it can inhibit the secretion of ACTH, or weaken the reaction of the cells secreting ACTH in the adenohypophysis to CRH, so as to reduce the secretion of glucocorticoid, so as to maintain the relatively stable content of glucocorticoid in the blood for the needs of the body.There may also be short loop negative feedback regulation between ACTH and CRH.In short, hypothalamus pituitary gland-adrenal glandThe three parts of cortex form an efficient functional axis.
In stress response,central nervous systemHypothalamus adenohypophysis-adrenal glandThe activity of cortical functional axis was strengthened.GlucocorticoidThe secretion increases rapidly. At this time, the negative feedback regulation of glucocorticoid temporarily fails, which is typicalopen loopThe mechanism of regulation and negative feedback failure is unknown.
It should be pointed out that long-term high-dose administration of exogenous glucocorticoid can feedback inhibit the adenohypophysisSecretory cellActivities that can eventually lead to selfadrenal glandAtrophy of cortex without hormone secretion.
Hyperfunction
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One or moreadrenal glandOversecretion of corticosteroids produces different clinical syndromes. OverproductionandrogenResults in the masculinization of the adrenal gland;GlucocorticoidHypersecretionCushing syndrome；Excessive aldosterone productionAldosteronismThese syndromes often overlap. Hyperadrenal function can be compensatory, such as congenital adrenal hyperplasia, or due toAcquirabilityHyperplasia, adenoma or adrenal carcinoma
Any syndrome, congenital or acquired, with excessadrenal glandandrogenIt can cause masculinization
Symptoms and signs:
The symptoms and signs depend on the sex and age of the patient at the beginning of the disease, and women are more obvious than men. Adult women,adrenal glandMasculinity can be attributed toAdrenal hyperplasiaandadrenal tumors The symptoms and signs of both diseases include hirsutism, alopecia,acne, the sound is low and dull,amenorrhoea,Uterine atrophy, clitoral hypertrophy,Breast reductionAnd muscle increase. Sexual desire can increaseHirsutismIt can be the only sign of mild cases
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adrenal glandCT and MRI are used to exclude tumors as the cause of masculinity. If tumors are foundX-rayOr ultrasound localization for small needle aspiration biopsy can obtain a lot of data
adrenal cortex
TardinessMasculinizationadrenal glandHyperplasia is a variant of congenital adrenal hyperplasia, both due tocortisolDefect of precursor hydroxylation in urineDHEAAndsulfate(DHEAS) increased;The excretion of pregnanetriol is often increased, andUrinary free cortisolDecrease. Plasma DHEA, DHEAS,17 hydroxyprogesterone,testosteroneandAndrostenedioneincrease.dexamethasone0.5mg is taken orally every 6 hours, and DHEA and progesterone triol inhibition can confirm the diagnosis. Dexamethasone 0.5mg~1mg should be taken orally before sleep, but even at such a small dose, Cushing's syndrome can also appear in some patients. It is also availableHydrocortisone(25mg/d) orprednisone(5-10mg/d) treatment. Although most symptoms and signs of masculinity disappear, the improvement of hirsutism and alopecia is slow, the tone is still low and dull, and pregnancy can be affected
Andadrenal glandDifferent hyperplasia, male adenoma orAdenocarcinomaDexamethasone does not inhibit or only partially inhibitsandrogenExcretion. The location of the tumor can be located by CT. Treatment requires adrenalectomy. Some tumors secrete excessive androgen andcortisolCausing Cushing's syndrome with ACTH secretion inhibition and contralateral adrenal atrophy. If so, hydrocortisone should be given before and after surgery, as described below. Mild hirsutism and masculinizationHypomenorrheaOr the increase of plasma testosterone can be seen in the polycystic ovary syndrome
adrenal glandcortexHyperfunctionCan beACTHDepends on or does not rely on ACTH regulation, such as due toAdrenal cortical adenomaOr cancercortisol. High physiological doseExogenousCortisol or related synthesis similarmedicationIt inhibits the adrenal cortex function and has simulated non ACTH dependent hyperfunctionACTH dependencyAdrenocortical hyperfunctionYou can do this because: (1)the pituitaryHypersecretion of ACTH;(2) Non pituitary ACTH secreting tumors, such asSmall cell carcinoma of lung(Ectopic ACTH syndrome)；Or (3) exogenous ACTH. The term Cushing's syndrome is applied to the clinical manifestations of cortisol excess caused by any reason (whatever the reason)adrenal glandHypercortical function is calledCushing disease, which means there are unique physiological abnormalities. Patients with Cushing's disease can be pituitary basophils orChromophobe cellTumor.
Morning (6~8 o'clock)Plasma cortisolNormal range: 5~25 μ g/dl (138~690nmol/50) And then gradually decline to evening (after 6 o'clock)<10 μ g/dl (L). Patients with Cushing's syndrome, usually in the morningcortisolIncrease, lack of normal cortisol production, decrease in the daytime, so that the plasma cortisol is higher than normal at night, and the total amount of cortisol production in 24 hours is increased. A single plasma cortisol sample may be difficult to explain becausePulsatile secretionIt causes a wide range of normal values. CongenitalCorticosteroid binding globulinIncrease patients, plasma cortisol may have a false increase, but these patients have normalCircadian rhythmUrinary free cortisol in patients with Cushing's disease, the best urine excretion test [the normal value is between 20~100 μ g/24h (55.2~276nmol/24h)], the increase in patients with Cushing's disease is more than 120 μ g/24h (>331nmol/24h),ObesityOnly slight increase<150 μ g/24h (<414nmol/24h)
Traditional dexamethasone test, dexamethasone 1mg, taken orally at 11~12 o'clock in the evening, and determined at 7~8 o'clock in the next morningPlasma cortisolThis method can be used as screening for Cushing's syndrome. For most normal people, the morning plasma cortisol is ≤ 5 μ g/dl (≤ 138 nmol/L), while for most non pituitary Cushing's syndrome patients, the morning cortisol level is at least 9 μ g/dl (248 nmol/L), and the plasma cortisol level is maintained at the initial level
When dexamethasone 0.5mg is taken once every 6 hours for 2 days (small dose), the ACTH secretion of normal people is inhibited. As a result, the urinary free cortisol drops to 50% or lower than that before taking the drug, but some patients drop to ≤ 10 μ g/24h (<27.6nmol/24h) the next day. In patients with Cushing's disease, the urinary free cortisol will not be reduced normally compared with the inhibition of dexamethasone. When dexamethasone 2mg is given once every 6 hours for 2 days (large dose)The urinary free cortisol of patients with Cushing's disease is often reduced by at least 50% compared with the basic value, because the disease depends on pituitary ACTH
adrenal glandTumor patients,cortisolGenerate independent ACTH, so dexamethasone has noinhibition. Patients with ectopic ACTH syndrome, nonPituitary tumorACTH production is almost always not affected by dexamethasone, so urinesteroidThe dexamethasone test can distinguish pituitary abnormalities from other types of Cushing's syndrome
The more accurate modified method is dexamethasone 1mg/h intravenousContinuous infusion7 hoursCushing diseaseThe patient's plasma cortisol decreased by at least 7 μ g/dl,adrenal glandPatients with tumor or ectopic ACTH syndrome had no responseDexamethasone inhibition testCan beLi FupingTherefore, this test is not helpful for diagnosis of patients taking this drug
midnightMepyrone testUsed to determine Cushing's syndromeEtiological diagnosisPituitary dependencyCushing diseasePatient, plasma 11 deoxygenationcortisolSignificantly increased, whileadrenal glandNo increase in patients with tumor or ectopic ACTH syndrome. Total steroid production (due to meperidone block11 Deoxycortisol11 hydroxylation) must be measured. Therefore, the determination of cortisol and 11 deoxycortisol found an increase in total steroids, rather than 11 deoxycortisol replacing cortisol in plasma
The less commonly used method for evaluating Cushing's syndrome isACTH stimulation test. ACTH50u intravenous infusion for 8 hours,Cushing diseaseThe urine cortisol of the patients increased 2-5 timesendogenousExcessive ACTH excitation, thus showing bilateraladrenal glandHyperplasia and over reaction to ACTH. About 50% of patients with adrenal adenoma will have obvious ACTH excitation, and sometimes significant plasma and urinary cortisol increase. Adrenal cancer generally has no reaction to ACTH
Pituitary microadenomaUsually, CT can find it, but MRI is better, especially gadolinium can increase the high resolution. Even with this technology, some microadenomas are still difficult to find. In some cases, although there is obvious excessive ACTH, it cannot be foundHistologyAbnormal
Differential diagnosis:
As suggested by the dexamethasone testadrenal glandFor tumors or ectopic ACTH syndrome, the plasma ACTH concentration can be measured. For ectopic ACTH syndrome, the plasma ACTH is significantly increased (usually>200pg/ml), and Cushing's syndrome caused by adrenal adenoma is so low that it is difficult to detect, unless the rare case is that ACTH secretes adrenal tumors. Patients with Cushing's disease usually have moderate to high plasma ACTH levels (75~200pg/ml)The laboratory results can also support that ectopic ACTH as the cause of Cushing's syndrome, including: low blood K (L) and HCO3 ->30mEq/L, 9AM serum cortisol>200 μ g/dl (>5520nmol/L), urinary free cortisol excretion>450 μ g/24h (>1242nmol/24h)
CRHTest (see aboveAddison's disease(experimental examination) can usually detect abnormal ACTH adrenocortical hyperfunctionadenomaHypersecretion (no response in this test) and pituitary typeCushing diseaseHowever, this test can mislead the diagnosis because there is overlap between normal and abnormal reactions. When combined with positive dexamethasone test, it has the greatest value
After the diagnosis of adrenal hyperfunction, the evaluation of Cushing's syndrome should also include CT but better MRIthe pituitaryExamination. If the existence of the tumor or the location of the pituitary tumor is uncertain, the most useful method is to give the patient CRH1 μ g/kg, and extract the bilateral sublithal before and after the medicationvenous sinusThe blood ACTH level was measured at the same time. Normal bilateral reactions were equal. Before excitation, the ACTH level in the blood from the tumor venous sinus was higher than that in the tumor free side, and there was a greater response to CRH. The increase of ACTH on both sides of patients with ectopic ACTH was equal, and there was no response to CRH. In addition, tumors produced by non pituitary ACTH must be carefully searched. The patients were given radioactive iodizationcholesterolLater, adrenal scanning can differentiate hyperplasia from adenoma or cancer. However, if biochemical experiments indicate the existence of adrenal tumors, adrenal area CT (MRI is not better than CT at this time) is the first choice
Children and adultsdiagnostic method Same as the standard, except that pregnant women prefer MRI in order to avoid radiation
Treatment is aimed atPituitary glandoradrenal glandCortical hyperfunction. The correct method depends on the basic disease
At the beginning, patients should generally be given appropriate potassium andhigh proteinSupport, if the clinical condition is seriousAminophenidate(250mg orally twice a day) or ketoconazole (400mg/d increased to the maximum dose of 1200mg/d) to block steroid secretion is more reasonable. When too much ACTH comes from the pituitary glandstandard method It is to carry out sphenoid pituitary exploration. If a tumor is found, it will be surgically removedsurgical operationThere are certain requirements, which should be carried out in an experienced center. The success rate of the operation is about 70%, preferably a microadenoma with a diameter of less than 1cm. About 20% of tumors recur, and large tumors and small tumors are more likely to recur. Recurrent tumors are often re operated successfully. Pregnancy is not a counter indication of surgery
kidney
If the tumor is not found, some doctors advocatethe pituitaryResection, but most people think that the next step should be pituitary hypertensionradiotherapy(40~50Gy). pituitary irradiation in children can reduceGHSecretion, occasionally causing puberty precocity. Some centers are heavyParticle beam irradiation(about 100Gy) is usually successful, and the reaction to radiation may take several months. Bilateraladrenal glandResection is only given to patients with pituitary adrenocortical hyperfunction who do not respond to pituitary exploration (with possible adrenalectomy) and radiotherapy (usually pituitary function returns to normal). Adrenalectomy requires lifelong steroidsAlternative treatmentAs required by primary adrenal failure
It has also developed intoNelson syndromeThe serious danger of Cushing's diseaseadrenal glandAfter excisionincidence rateAbout 5%~10%. If the patient receives pituitary radiotherapy, the risk is reduced. At the time of surgery, the patient is over 35 years old, and the risk is very low. Nelson's syndrome, the pituitary gland continues to increase, resulting in an increase in ACTH and β - MSH, leading to severe hyperpigmentation. Although radiotherapy can prevent the pituitary gland from continuing to grow in these patients, many patients also need pituitary resection. The indication for pituitary resection is the same as other pituitary tumors -- the size is increased,Erosion of surrounding tissuesVisual field defect, oppress hypothalamus or otherscomplication. Routine irradiation is usuallyHypophysectomyin the future.
adrenal glandThe tumor can be removed surgically. The patient must be supplemented during and after the operationcortisolBecause the nontumorous adrenal cortex has shrunk and been suppressed. Benign adenoma can be successfully removed by laparoscope. Multinodular adrenal hyperplasia requires bilateral adrenalectomy. Even if it is presumed that it has been completely removed, recurrence still accounts for about 1/3 of patients. If possible, the treatment of ectopic ACTH syndrome should remove the non pituitary tumor that produces ACTH. However, most patientsTumor spreadIt is impossible to resect. Adrenal inhibitors, such as meperidone 250mg, taken orally 4 times a day in combination with albendazione 250mg, taken orally twice a day, do not exceed 2g/d at most, or bothChlorobenzene dichloroethane(O,P′-DDD）0.5 Oral administration 4 times a day, up to 8-12g/d, usually can control the severity of adrenal cortex hyperfunctionMetabolic disorder(such as hypokalemia). When taking dichlorobenzene dichloroethane, hydrocortisone 20mg/d should be taken at the same time to prevent the complete disappearance of the secretion of corticosteroids in patients. However, ketoconazole (400~1200mg/d) can probably best block the synthesis of steroids. Although there is a risk of liver toxicity, like dichlorobenzene dichloroethane, it can cause the symptoms of Addison's disease. In addition, corticosteroid receptors canMifepristone(RU486) block. This increases the plasma cortisol level, but blocks the steroid action. Sometimes, ectopic ACTH syndrome tumors have long-term effectsSomatostatinAnaloguesReact, such asoctreotide(Octreotide) 100~125 μ g, 3 times a daysubcutaneous injectionIf octreotide is used for more than 2 years, close follow-up is required because it can be mildgastritis,gallstone,cholangitis,jaundiceandVitamin B12Malabsorption.
Aldosteronism
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becausealdosteroneClinical syndrome caused by excessive secretion
Aldosterone isadrenal glandProduce the most powerfulMineralocorticoid, can cause sodium retention and potassium excretion. In the kidney, aldosterone causes distalRenal tubuleSodium enters tubular cells and exchanges with potassium and hydrogen. The same effect can be seen insalivary glands,Sweat glandAnd intestinal mucosal cells and intracellular and extracellular fluids
PrimaryAldosteronismbecauseadrenal glandCortical glomerular zoneadenoma, usually unilateral, rareadrenal glandCancer or hyperplasia. Adenoma is extremely rare in children, but sometimes the syndrome is adrenal cancer orAdrenal hyperplasiaDue to the lack of 11 β-hydroxylaseThe clinical features of congenital adrenal hyperplasia also mimic this syndrome. Hyperaldosterone andHypokalemiaThe difference with aldosteronism is nonehypertension.
Symptoms and signs:
Hypersecretion of aldosterone can lead to hypernatremia, hyperchloremia, hypervolemia and hypokalemia, and the clinical manifestation is paroxysmal weakness,Paresthesia, transient paralysis andTetany.diastolic pressureHyperkalemic and hypokalemic nephropathy withPolyuriaIt is common to be thirstyastumour, high sodium intake (>10g/d), aldosterone excretion usually>200 μ g/d. Sodium deprivation can cause potassiumretention. personality change, hyperglycemia,Urine sugarOccasionally. In many cases, the only manifestation is mildModerate hypertension.
diagnosis:
Spironolactone testHelps diagnose. GiveSpironolactone200~400mg/d, oral for 5~8 weeks, can reverse the diseaseclinical manifestation, including hypertension (nonaldosteroneThe increase of hypertension can rarely be reversed). Plasma renin measurement is helpful for diagnosis. Plasma renin level is measured when the patient lies on his back in the morningFurosemide80 mg, and then the patient stood upright for 3 hours, and then the plasma renin was measured. The plasma renin of normal people increased significantly after standing upright, andAldosteronismNo increase in patients. About 20%essential hypertensionThe patient does not necessarily have high aldosterone but low renin, and has no reaction to upright positionPlasma aldosterone(Peripheral veinoradrenal glandVenous intubation) can be helpful. Therefore, the diagnosis depends on the confirmation of high aldosterone secretion in blood and urine and the absence of increased plasma renin in upright positionExpansion of extracellular fluid volumeAnd potassium abnormalityCTA small adrenal adenoma that often shows these diseasesMRIIt does not improve the diagnostic ability
treatment:
oncePrimaryaldosteroneDefinite diagnosis, bilateral explorationadrenal glandBecause there may be multiple adenomas. It may be necessary to cut the gland to look for adenoma. ObviouslyAldosteronismWhen a single adenoma is confirmedGood prognosisThese cases may requirelaparoscopeExcision of adenoma. ExcisionAldosterone adenomaAfter that, the patients' blood pressure decreased by about 50%~70%complete remission Although most patients with adrenal hyperplasia and aldosteronism have lowered their blood pressure, about 70% of them are still hypertensivePrimary aldosteronismIt can usually be controlled by spironolactone. The initial dose is 300mg/d, and gradually reduces to the maintenance dose in one month, usually 100mg/d, orPotassium canylateIn addition, about half of the patients need antihypertensive treatment (see Section 119). Bilateral adrenalectomy is rarely required. It is difficult to diagnose and define normal blood potassium aldosteronism,SurgeryProbing may be futile
Secondary aldosteronism,adrenal glandThe increase of aldosterone production is stimulated by the adrenal gland, which is similar to the primary disease, and is related to hypertension and edema (such asheart failure,Cirrhotic ascites,Nephrotic syndrome）The secondary aldosteronism in the acute phase of hypertension is believed to be caused by the secondary kidneyvasoconstrictionAldosteronism is also seen in obstructive renal artery hypertension (e.gatherosclerosisAnd stenosis). This is due to the reduction of renal blood flow on the diseased side. It is commonly seen in edematousHypovolemiain especialdiureticExcitement during treatmentRenin angiotensin systemWith excessive aldosterone secretion, the secretion rate can be normal in heart failure, but the liver blood flow and aldosterone metabolism are reduced, resulting inBlood circulationhormoneThe level increases
Iadrenal glandTuberculosis: Only bilateral adrenal tuberculosis, most of the adrenal tissue is destroyed, can clinical symptoms appear.It is often accompanied by tuberculosis in the lung, bone or other parts.In the 1950s, it accounted for aboutadrenal glandIn recent years, half of the patients with cortical hypofunctiontuberculosisControlled and gradually reduced.
1、 The slow onset of disease may not be noticed until many years later.Occasionally, some cases are induced by infection, trauma, surgery and other stressAdrenal crisisWas found clinically.
2、 Pigmentation skin and mucosa pigmentation, mostlyDiffuse, so as to expose the parts, frequently rubbing parts andFingernailRoot, scarAreola、External genitalia, perianal, gingival, oral mucosa, conjunctiva are obvious.The reason for pigmentation is that when glucocorticoid is reducedMelanocyteStimulating hormone (MSH) andadrenal glandCorticosteroid (ACTH) secretingFeedback suppressionDecrease.Some patients may have flakesDepigmentationZone.secondaryadrenal glandThe levels of MSH and ACTH in patients with cortical hypofunction were significantly reduced, so there was no pigmentation.
4、 Gastrointestinal symptoms such asAnorexia, nausea, vomiting, upper abdomen, right lower abdomen or no positioningabdominal pain, sometimes with diarrhea or constipation.She likes high sodium diet.Often accompanied by emaciation.Digestive tract symptoms are more common in patients with a long course of disease and serious illness.
VIhypoglycemiaDue to the lack of insulin antagonists andGastrointestinal disordersThe patient's blood sugar is often low, but due to the slow development of the disease, the patient can tolerate it and the symptoms are not obvious.Only hunger, sweatingheadache, weak and uneasy.In severe cases, tremorBlurred vision, diplopiainsane, even convulsions, coma.This disease is particularly sensitive to insulin, and even a small dose of injection can cause severe hypoglycemic reaction.
VIII.adrenal glandLow resistance of crisis patients, anyIrritabilityLoads such as infection, trauma, surgery and anesthesia can induce acute adrenal cortical hypofunction crisis.
（1） Urinary 17 hydroxycorticosteroids (17OHCS) and 17 ketocorticosteroids (17KS)DischargeBelow normal.The degree of reduction andadrenal glandCortex function is parallel.
(II)Plasma cortisolMeasured, most of them were significantly reduced, and the circadian rhythm disappeared.
（3） ACTH excitation test This test is an inspectionadrenal glandFunctional reserve of cortex.Light weight can be foundChronic adrenocortical hypofunctionPatients and differential diagnosis between primary chronic adrenocortical hypofunction and secondary chronic adrenocortical hypofunction.
3、 Primary determination of plasma ACTH basic valueadrenal glandThe level of ACTH in patients with cortical hypofunction was significantly higher, more than 55pmol/L (250pg/ml), often between 88-440pmol/L (400-200pg/ml) (the normal value was 1.1-11pmol/L, that is, 5-50pg/ml), while the plasma ACTH concentration in patients with secondary adrenal cortical hypofunction was extremely low.
4、 The cause of tuberculosis was examinedadrenal glandIt is possible to seeCalcification focusThere may also be tuberculosis lesions in other tissues and organs.In itselfImmunityadrenal glandAdrenal cortex antibody may be detected in the serum of patients with cortex destruction, and patients are often accompanied by otherAutoimmune diseaseandEndocrine glandLow function.Transferabilityadrenal glandPrimary cancer may be found in cancer patients.
treatment:
1、 Take high sodium diet during basic treatment, and take alternative treatmenthydrogenationKautesong20-30mg daily or 5-7.5mg prednisone should be taken in the morningTotal dose2/3, take 1/3 in the afternoon if fatigue, tiredness andHyponatremia, small dosage can be addedMineralocorticoid, such as 9 α - fludrocortisone 0.2mg daily or intramuscular injection of trimethylamine monthlyDeoxycorticosterone acetate125mg 。
2、 The treatment of acute cortical functional crisis increases hydrocortisone by about 50mg every day during mild stress. Those who cannot take hydrocortisone orally canIntravenous dripAdministration。Severe acuteadrenal glandCrisis, which is more life threatening, must be rescued in time. ①Add salt water, quickly add salt water in the first two days, 2~3L per day. ②Glucocorticoid, immediateintravenous injectionphosphoric acidHydrocortisone orSuccinylHydrocortisone 100mgPlasma cortisolThe concentration reaches the level of normal people when severe stress occurs.Every 6 hours thereafterIntravenous drip100mg, gradually reduced on the third day. After vomiting stops, hydrocortisone 50~60mg/d can be taken orally.9 α - can be addedFluhydrocortisone。
IIIEtiological treatmentSuch as immunosuppressant, anti tuberculosis treatment, etc.
Hyperplasia
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This disease is due tothe pituitaryOr ectopic promotionadrenal glandCorticosteroid celladenomaOversecretion of adrenocorticotropic hormone causes hyperplasia and hypertrophy of adrenal cortex.
Adrenal cortical hyperplasia can be divided into two types:
(I)NodularHyperplasia: bilateraladrenal glandMultiple hyperplasia can be seen inside or outside the capsule or in fatTubercleMm to 2.5 cm in diameter.The arrangement and shape of the cells in the nodules are similar to the spherical zone orFascicular bandSimilar, common and largeLipofuscinThe nodules are brownish brown.
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（2） Diffuse hyperplasia: usually bilateral, singleadrenal glandIts weight is more than 5g and can reach more than 8g. Its cortex thickness can reach 2mm and its edge is blunt.The changes of cortical globular zone of diffuse hyperplasia were mostly not obvious, or only increased layers were seen;Fascicular bandThe proliferation is obvious, and the cells are enlarged,CytoplasmlipidThe number of vacuolated zona fasciculata cells was increased, and the common cells extended into the zona reticularis in tongue shape.Most patients appear clinicallyadrenal glandCortical hyperfunction due tocortisolExcessive secretion will lead to protein alienation and secondary adiposis.performanceCushing syndrome, appearsCentripetal obesity、Full moon face, shoulder and back hypertrophyMuscular atrophy、osteoporosis, skin thinning and purple lines, hairyacne, hypertension, impaired glucose tolerance, menstrual disorders andSexual functionDecline, etc.
All types of congenitaladrenal glandCharacteristics and treatment of cortical hyperplasia:
Caused by different kinds of enzyme defectsAdrenal cortical hyperplasia, will affect thehormoneThe synthesis process (see Figure 10) blocks the synthesis of this kind of hormoneIntermediate productExcessive production, resulting in corresponding clinicalsign。
21 hydroxylase deficiency is the most common type, mainly glucocorticoid andMineralocorticoidlack,androgenToo many.The treatment is to givePhysiological requirementGlucocorticoid and mineralocorticoid.Children can take hydrogen orally by the age of 5Cortisone20-25 mg/day, maintain urine 17 ketosteroid level at 6.94-10.41 μ gmole/Day.For children aged 6-12 years, the amount of hydrocortisone can be increased to 25-50 mg/day to maintain the urine 17 keto group alcohol at 13.88-27.76 μ mol/day.Or take corresponding prednisone orPrednisolone。Be careful not to overdose, causing iatrogenicCushing Syndrome.Such as hypertensionGlucocorticoidIn case of adverse reaction, it is necessary to carefully consider whether to conduct bilateral treatmentadrenal glandExcision.
Efficacy: This prescription is used to treat various late stageMalignant tumor140 cases.Among them, 34 patients survived 1-3 years;42 cases in 3-5 years;40 cases in 5-10 years;24 cases over 10 years;The longest survival time is 16 years.
